Schönlein-Henoch purpura (PSH) (synonyms: Acute infantile hemorrhagic edema; allergic purpura; allergic vasculitis; anaphylactoid purpura; arthritis in Schoenlein-Henoch purpura; arthritic purpura; arthropathy in Schoenlein-Henoch purpura; autoimmune vasculitis; bacterial purpura; gangrenous purpura; gangrenous purpura without consumption coagulopathy; Brain purpura; glomerular disease in Schoenlein-Henoch purpura; glomerulonephritis in Schoenlein-Henoch purpura; hemorrhagic nonthrombocytopenic purpura; Henoch-Schoenlein disease; Henoch-Schoenlein syndrome; Henoch-Schoenlein purpura; brain purpura; idiopathic nonthrombocytopenic purpura; IgA vasculitis; Immune complex vasculitis; immunovascular reaction; immune vasculitis; infectious purpura; capillary hemorrhagic toxicosis; leukocytoplastic vasculitis; malignant purpura; Schoenlein-Henoch disease; Schoenlein-Henoch disease; necrotizing vasculitis; peliosis; peliosis rheumatica; purpura abdominalis; purpura allergica; Purpura anaphylactoides; Purpura with visceral symptoms; Purpura nervosa; Purpura rheumatica; Purpura symptomatica; Rheumatoid purpura; Schoenlein-Henoch disease; Schoenlein-Henoch syndrome; Schoenlein-Henoch purpura; Seidlmayer cocard purpura; Toxic purpura; Vasculitis allergica; Vascular purpura; ICD-10 D69. 0: Purpura anaphylactoides) is an immunologically mediated vasculitis (vascular inflammation) of the capillaries and pre- and postcapillary vessels. Deposits of IgA1 immune complexes (immune complex = allergen + antibody) occur. Both superficial skin vessels (vasculitits allergica superficialis) and deeper skin vessels (vasculitits allergica profunda) can be affected. As a multisystem disease, Schönlein-Henoch purpura preferentially affects the skin, joints, intestines, and kidneys.
Schönlein-Henoch purpura belongs to the group of vasculitides (vascular inflammation) and here to the non-ANCA-associated/immune complex small vessel vasculitis. It is increasingly referred to as IgA vasculitis (IgAV).
According to the cause, Schönlein-Henoch purpura is divided into a primary and a secondary form. The primary form is often triggered by infections or drugs. The secondary form is caused by another disease. In about 50% of cases, the cause of Schönlein-Henoch purpura is not identifiable (idiopathic Schönlein-Henoch purpura).
Sex ratio: Boys are slightly more frequently affected than girls.
Frequency peak: The frequency peak of Schönlein-Henoch purpura is almost exclusively in childhood. Especially children of preschool age are affected by the disease. Schönlein-Henoch purpura is the most common vasculitis of the young child and school-aged child.
The incidence (frequency of new cases) is approximately 15-25 cases per 100,000 population per year.
Course and prognosis: Primary Schönlein-Henoch purpura is acute, while the secondary form is chronic and relapsing.Often, Schönlein-Henoch purpura is accompanied by arthritis (inflammation of the joints). The occurrence of glomerulonephritis (inflammation of the glomeruli (renal corpuscles) of the kidneys) is also possible. After years, chronic renal insufficiency may develop, so long-term monitoring of the disease is required. In addition, especially in adults, Schönlein-Henoch purpura may recur (relapse) after remission (disappearance of disease symptoms) has been achieved.In children, the disease usually heals spontaneously (by itself) and without consequences. In adults, complications are more common or more severe, worsening the prognosis.